| Post Translational Modifications | Phosphorylated at Ser-64 by ATM in response to DNA damage, promoting its interaction with histone H2AX and localization to sites of DNA double-strand breaks. |
| Function | RNA chaperone that plays a key role in telomere maintenance and RNA localization to Cajal bodies. Specifically recognizes and binds the Cajal body box (CAB box) present in both small Cajal body RNAs (scaRNAs) and telomerase RNA template component (TERC). Essential component of the telomerase holoenzyme complex, a ribonucleoprotein complex essential for the replication of chromosome termini that elongates telomeres in most eukaryotes. In the telomerase holoenzyme complex, required to stimulate the catalytic activity of the complex. Acts by specifically binding the CAB box of the TERC RNA and controlling the folding of the CR4/CR5 region of the TERC RNA, a critical step for telomerase activity. In addition, also controls telomerase holoenzyme complex localization to Cajal body. During S phase, required for delivery of TERC to telomeres during S phase and for telomerase activity. In addition to its role in telomere maintenance, also required for Cajal body formation, probably by mediating localization of scaRNAs to Cajal bodies. Also plays a role in DNA repair: phosphorylated by ATM in response to DNA damage and relocalizes to sites of DNA double-strand breaks to promote the repair of DNA double-strand breaks. Acts by recruiting the ubiquitin ligase RNF8 to DNA breaks and promote both homologous recombination (HR) and non-homologous end joining (NHEJ). |
| Protein Name | Telomerase Cajal Body Protein 1Wd Repeat-Containing Protein 79Wd40 Repeat-Containing Protein Antisense To Tp53 GeneWrap53beta |
| Database Links | Reactome: R-HSA-171319Reactome: R-HSA-390471 |
| Cellular Localisation | NucleusCajal BodyChromosomeTelomereReleased From Telomerase Rna Template Component (Terc) In Mitotic Cells Coincident With Delocalization From Cajal BodiesIn Response To Dna DamageLocalizes To Sites Of Dna Double-Strand Breaks Following Phosphorylation By Atm |
| Alternative Protein Names | Telomerase Cajal Body Protein 1 proteinWd Repeat-Containing Protein 79 proteinWd40 Repeat-Containing Protein Antisense To Tp53 Gene proteinWrap53beta proteinWRAP53 proteinTCAB1 proteinWDR79 protein |
Information sourced from Uniprot.org
